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Erschienen in: European Journal of Pediatrics 5/2009

01.05.2009 | Review

Clinical practice: pulmonary hypertension in children

verfasst von: Daniel De Wolf

Erschienen in: European Journal of Pediatrics | Ausgabe 5/2009

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Abstract

Introduction

Pulmonary arterial hypertension is a rare disorder in childhood, the two most common types being idiopathic pulmonary arterial hypertension and pulmonary hypertension associated with congenital left-to-right shunt lesions, together accounting for almost 90% of cases.

Discussion

The clinical presentation of idiopathic pulmonary arterial hypertension (familial and non familial) is essentially non-specific and varies with age. Pulmonary vasoreactivity testing identifies responders and non-responders. Responders are treated with calcium channel blockers and have a better prognosis. Non-responders have a very limited survival beyond diagnosis if not treated with more selective pulmonary arterial vasodilators. Prostacyclin, endothelin receptor antagonists and phosphodiesterase-5 inhibitors improve haemodynamics, functional class and exercise tolerance and delay deterioration. Patients with congenital left-to-right shunts and irreversible pulmonary arterial hypertension leading to Eisenmenger’s syndrome have multiple organ disease. Despite a very pronounced exercise intolerance, their clinical course is rather stable with survival up to 40–60 years, depending on the complexity of their underlying cardiac defect. Treatment is based on general measures along with the same three types of selective pulmonary vasodilators as in idiopathic pulmonary arterial disease. Improvement in haemodynamics, functional class and exercise tolerance are comparable for both patient groups.

Conclusion

Pulmonary hypertension in children is idiopathic or associated with congenital heart disease in the majority of patients. Treatment with new selective pulmonary vasodilators offers haemodynamic and functional improvement.
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Literatur
1.
Zurück zum Zitat Avila W, Grinberg M, Snitcowsky R et al (1995) Maternal and fetal outcome in pregnant women with Eisenmenger’s syndrome. Eur Heart J 16:460–464PubMed Avila W, Grinberg M, Snitcowsky R et al (1995) Maternal and fetal outcome in pregnant women with Eisenmenger’s syndrome. Eur Heart J 16:460–464PubMed
2.
Zurück zum Zitat Beghetti M, Hoeper M, Kiely D et al (2008) Safety experience with bosentan in 146 children 2-11 years old with pulmonary arterial hypertension: results from the European Postmarketing Surveillance program. Pediatr Res 64:200–204PubMedCrossRef Beghetti M, Hoeper M, Kiely D et al (2008) Safety experience with bosentan in 146 children 2-11 years old with pulmonary arterial hypertension: results from the European Postmarketing Surveillance program. Pediatr Res 64:200–204PubMedCrossRef
3.
Zurück zum Zitat Boucek MM, Aurora P, Edwards LB et al (2007) Registry of the International Society for Heart and Lung Transplantation: tenth official pediatric heart transplantation report. J Heart Lung Transplant 26:796–807PubMedCrossRef Boucek MM, Aurora P, Edwards LB et al (2007) Registry of the International Society for Heart and Lung Transplantation: tenth official pediatric heart transplantation report. J Heart Lung Transplant 26:796–807PubMedCrossRef
4.
Zurück zum Zitat Chau E, Fan K, Chow W (2007) Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol 120:301–305PubMedCrossRef Chau E, Fan K, Chow W (2007) Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol 120:301–305PubMedCrossRef
5.
Zurück zum Zitat D’Alonzo G, Barst R, Ayres S et al (1991) Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med 115:343–349PubMed D’Alonzo G, Barst R, Ayres S et al (1991) Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med 115:343–349PubMed
6.
Zurück zum Zitat Daliento L, Somerville J, Presbitero P et al (1998) Eisenmenger syndrome: factors relating to deterioration and death. Eur Heart J 19:1845–1855PubMedCrossRef Daliento L, Somerville J, Presbitero P et al (1998) Eisenmenger syndrome: factors relating to deterioration and death. Eur Heart J 19:1845–1855PubMedCrossRef
7.
Zurück zum Zitat Diller GP, Gatzoulis M (2007) Pulmonary vascular disease in adults with congenital heart disease. Circulation 115:1039–1050PubMedCrossRef Diller GP, Gatzoulis M (2007) Pulmonary vascular disease in adults with congenital heart disease. Circulation 115:1039–1050PubMedCrossRef
8.
Zurück zum Zitat Diller GP, Dimopoulos K, Broberg C et al (2006) Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case–control study. Eur Heart J 27:1737–1742PubMedCrossRef Diller GP, Dimopoulos K, Broberg C et al (2006) Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case–control study. Eur Heart J 27:1737–1742PubMedCrossRef
9.
Zurück zum Zitat Duffels M, Engelfriet P, Berger R et al (2007) Pulmonary arterial hypertension in congenital heart disease/an epidemiologic perspective from a Dutch registry. Int J Cardiol 120:198–204PubMedCrossRef Duffels M, Engelfriet P, Berger R et al (2007) Pulmonary arterial hypertension in congenital heart disease/an epidemiologic perspective from a Dutch registry. Int J Cardiol 120:198–204PubMedCrossRef
10.
Zurück zum Zitat Fasnacht M, Tolsa J, Beghetti M (2007) The Swiss registry for pulmonary arterial hypertension: the paediatric experience. Swiss Med Wkly 137:510–513PubMed Fasnacht M, Tolsa J, Beghetti M (2007) The Swiss registry for pulmonary arterial hypertension: the paediatric experience. Swiss Med Wkly 137:510–513PubMed
11.
Zurück zum Zitat Galie N, Beghetti M, Gatzoulis M et al (2006) Bosentan therapy in patients with Eisenmenger syndrome/a multicenter, double-blind, randomised, placebo-controlled study. Circulation 114:48–54PubMedCrossRef Galie N, Beghetti M, Gatzoulis M et al (2006) Bosentan therapy in patients with Eisenmenger syndrome/a multicenter, double-blind, randomised, placebo-controlled study. Circulation 114:48–54PubMedCrossRef
12.
Zurück zum Zitat Galie N, Ghofrani H, Torbicki A et al (2005) Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 353:2148–2157PubMedCrossRef Galie N, Ghofrani H, Torbicki A et al (2005) Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 353:2148–2157PubMedCrossRef
13.
Zurück zum Zitat Galie N, Manes A, Palazzini M et al (2008) Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger’s syndrome. Drugs 68:1049–1066PubMedCrossRef Galie N, Manes A, Palazzini M et al (2008) Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger’s syndrome. Drugs 68:1049–1066PubMedCrossRef
14.
Zurück zum Zitat Harrison R, Berger R, Haworth S et al (2005) Transforming growth factor-beta receptor mutations and pulmonary hypertension in childhood. Circulation 111:435–441PubMedCrossRef Harrison R, Berger R, Haworth S et al (2005) Transforming growth factor-beta receptor mutations and pulmonary hypertension in childhood. Circulation 111:435–441PubMedCrossRef
15.
Zurück zum Zitat Haworth SG (2008) The management of pulmonary hypertension in children. Arch Dis Child 93:620–625PubMedCrossRef Haworth SG (2008) The management of pulmonary hypertension in children. Arch Dis Child 93:620–625PubMedCrossRef
16.
Zurück zum Zitat Humbert M, Sitbon O, Chaouat A et al (2006) Pulmonary Arterial Hypertension in France. Results from a National Registry. Am J Respir Crit Care Med 173:1023–1030PubMedCrossRef Humbert M, Sitbon O, Chaouat A et al (2006) Pulmonary Arterial Hypertension in France. Results from a National Registry. Am J Respir Crit Care Med 173:1023–1030PubMedCrossRef
17.
Zurück zum Zitat Humpl T, Reyes J, Holtby H et al (2005) Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation 111:3274–3280PubMedCrossRef Humpl T, Reyes J, Holtby H et al (2005) Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation 111:3274–3280PubMedCrossRef
18.
Zurück zum Zitat Maiya S, Hislop A, Flynn Y et al (2006) Response to bosentan in children with pulmonary hypertension. Heart 92:664–670PubMedCrossRef Maiya S, Hislop A, Flynn Y et al (2006) Response to bosentan in children with pulmonary hypertension. Heart 92:664–670PubMedCrossRef
19.
Zurück zum Zitat Mathai S, Girgis R, Fisher M et al (2007) Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 29:469–475PubMedCrossRef Mathai S, Girgis R, Fisher M et al (2007) Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 29:469–475PubMedCrossRef
20.
21.
Zurück zum Zitat Peacock A, Murphy N, McMurray J et al (2007) An epidemiological study of pulmonary arterial hypertension. Eur Respir J 30:104–109PubMedCrossRef Peacock A, Murphy N, McMurray J et al (2007) An epidemiological study of pulmonary arterial hypertension. Eur Respir J 30:104–109PubMedCrossRef
22.
Zurück zum Zitat Perloff J, Hart E, Greaves M et al (2003) Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension. Am J Cardiol 92:182–187PubMedCrossRef Perloff J, Hart E, Greaves M et al (2003) Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension. Am J Cardiol 92:182–187PubMedCrossRef
23.
Zurück zum Zitat Raymond R, Hinderliter A, Willis P et al (2002) Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 39:1214–1219PubMedCrossRef Raymond R, Hinderliter A, Willis P et al (2002) Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 39:1214–1219PubMedCrossRef
24.
Zurück zum Zitat Rich S, Dantzker D, Ayres S et al (1987) Primary pulmonary hypertension: a national prospective study. Ann Intern Med 107:216–223PubMed Rich S, Dantzker D, Ayres S et al (1987) Primary pulmonary hypertension: a national prospective study. Ann Intern Med 107:216–223PubMed
25.
Zurück zum Zitat Roberts K, McElroy J, Wong W et al (2004) BMPR2 mutations in pulmonary hypertension with congenital heart disease. Eur Resp J 24:371–374CrossRef Roberts K, McElroy J, Wong W et al (2004) BMPR2 mutations in pulmonary hypertension with congenital heart disease. Eur Resp J 24:371–374CrossRef
26.
Zurück zum Zitat Rosenzweig E, Ivy D, Widlitz A et al (2005) Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 46:697–704PubMedCrossRef Rosenzweig E, Ivy D, Widlitz A et al (2005) Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 46:697–704PubMedCrossRef
27.
Zurück zum Zitat Rosenzweig E, Kerstein D, Barst R (1999) Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 99:1858–1865PubMed Rosenzweig E, Kerstein D, Barst R (1999) Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 99:1858–1865PubMed
28.
Zurück zum Zitat Rubin L, Badesch D, Barst R et al (2002) Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 346:896–903PubMedCrossRef Rubin L, Badesch D, Barst R et al (2002) Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 346:896–903PubMedCrossRef
29.
Zurück zum Zitat Simonneau G, Galiè N, Rubin L et al (2004) Clinical classification of pulmonary hypertension. J Am Coll Cardiol 43(suppl 1):S5–S12CrossRef Simonneau G, Galiè N, Rubin L et al (2004) Clinical classification of pulmonary hypertension. J Am Coll Cardiol 43(suppl 1):S5–S12CrossRef
30.
Zurück zum Zitat The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology (2004) ESC guidelines. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. Eur Heart J 25(24):2243–2278CrossRef The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology (2004) ESC guidelines. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. Eur Heart J 25(24):2243–2278CrossRef
31.
Zurück zum Zitat van Loon R, Hoendermis E, Duffels M et al (2007) Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist? Am Heart J 154:776–782PubMedCrossRef van Loon R, Hoendermis E, Duffels M et al (2007) Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist? Am Heart J 154:776–782PubMedCrossRef
32.
Zurück zum Zitat Yuan J, Rubin L (2005) Pathogenesis of pulmonary arterial hypertension: the need for multiple hits. Circulation 111:534–538PubMedCrossRef Yuan J, Rubin L (2005) Pathogenesis of pulmonary arterial hypertension: the need for multiple hits. Circulation 111:534–538PubMedCrossRef
33.
Zurück zum Zitat Yung D, Widlitz A, Rosenzweig E et al (2004) Outcomes in children with idiopathic pulmonary arterial hypertension. Circulation 110:660–665PubMedCrossRef Yung D, Widlitz A, Rosenzweig E et al (2004) Outcomes in children with idiopathic pulmonary arterial hypertension. Circulation 110:660–665PubMedCrossRef
Metadaten
Titel
Clinical practice: pulmonary hypertension in children
verfasst von
Daniel De Wolf
Publikationsdatum
01.05.2009
Verlag
Springer-Verlag
Erschienen in
European Journal of Pediatrics / Ausgabe 5/2009
Print ISSN: 0340-6199
Elektronische ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-008-0920-x

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